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Desmoplastic Small Round Cell Tumor : Current Management and Recent Findings
Joint Authors
Dufresne, Armelle
Blay, Jean-Yves
Cassier, Philippe
Meeus, Pierre
Marec-Bérard, Perrine
Couraud, Laure
Alberti, Laurent
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-03-29
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults.
Patients typically present with symptoms of abdominal sarcomatosis.
Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma.
DSRCT is associated with a unique chromosomal translocation t(11:22) (p 13; q 12) that involves the EWSR1 and WT1 genes.
The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease.
Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation.
Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [90Y]Yttrium microsphere liver embolization.
American Psychological Association (APA)
Dufresne, Armelle& Cassier, Philippe& Couraud, Laure& Marec-Bérard, Perrine& Meeus, Pierre& Alberti, Laurent…[et al.]. 2012. Desmoplastic Small Round Cell Tumor : Current Management and Recent Findings. Sarcoma،Vol. 2012, no. 2012, pp.1-5.
https://search.emarefa.net/detail/BIM-492754
Modern Language Association (MLA)
Dufresne, Armelle…[et al.]. Desmoplastic Small Round Cell Tumor : Current Management and Recent Findings. Sarcoma No. 2012 (2012), pp.1-5.
https://search.emarefa.net/detail/BIM-492754
American Medical Association (AMA)
Dufresne, Armelle& Cassier, Philippe& Couraud, Laure& Marec-Bérard, Perrine& Meeus, Pierre& Alberti, Laurent…[et al.]. Desmoplastic Small Round Cell Tumor : Current Management and Recent Findings. Sarcoma. 2012. Vol. 2012, no. 2012, pp.1-5.
https://search.emarefa.net/detail/BIM-492754
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-492754